Biliary Atresia: Newborn Infants with a Rare Liver Disease Due to Blocked Bile Ducts
Every birth of a child is a miracle of life. The happiness it brings to the parents is immeasurable. But life also brings its own heartaches when the joy turns to sorrow and despair when harsh reality hits back. The child is born with Biliary Atresia.
Biliary Atresia is a very serious and rare liver disease of newborn infants. The cause is not known as to why this condition occurs in about one in 10,000 children. This condition is more common in Asian and African-American babies compared to Caucasian newborns. Biliary Atresia also occurs more commonly in girls than boys.
The first sign of Biliary Atresia is jaundice, a condition in which the infant exhibits a yellowish color of the skin and the eyes. Normally, there are healthy newborns that go through the jaundice phase in the first two weeks of their lives due to their liver, being still immature. But this condition disappears by the third to fourth week.
In the case of infants with Biliary Atresia, their jaundice worsens.
The human body has its own way of disposing whatever it does not need and here is where the important function of the liver enters the scene. It is important to note that the liver produces bile. Bile is a dark-green to yellowish fluid that does very important jobs. It serves as a “transport” for all the necessary substances needed in digesting the food and the absorption of fats and fat-soluble vitamins in the small intestines. And also has a role in helping the body disposes of the waste products, including bilirubin, which is a toxic breakdown product of hemoglobin and a yellow pigment in the blood.
In a normal and healthy liver, bile should smoothly flow through the many bile ducts into the small intestines and gallbladder, to do what is supposed to do. But in the case of Biliary Atresia, the bile ducts are blocked or maybe absent.
Problems arise when the waste products along with bilirubin are unable to get out and just back up in the liver. Jaundice happens when there is a high level of bilirubin in the blood, thus the yellowish color.
In a worst case scenario, liver damage may occur due to the inability of the bile to pass through the bile ducts. Toxic build up can cause scarring of the liver tissue. Eventually, the liver will cease to function normally leading to the necessity of a liver transplantation within the first 1 to 2 years of the child’s life.
Aside from jaundice, other symptoms shown by infants with Biliary Atresia are darkening of the urine and pale colored feces. Darkened urine is caused by the high levels of bilirubin in the blood that has spilled into the urine. While the pale colored feces is caused by the lack of bilirubin in the intestines.
The specific diagnosis of Biliary Atresia is done through a series of tests such as ultrasound of the abdomen and liver, liver scans and liver biopsy. Blood, urine and stool samples are also collected.
Treatments for Biliary Atresia is done through a surgery called Kasai Procedure. This procedure is developed by Professor Morio Kasai, a Japanese surgeon, in 1959. For this procedure to be a success, patients must not be over 90 days old. This process is done by removing the blocked bile ducts and being replaced by a loop of intestine, in order to drain the liver.
Unfortunately, the Kasai Procedure is not a 100% guarantee that the infant with Biliary Atresia is free from problems. Although the procedure may be an initial success, there is no guarantee that liver function and development is at its optimum. And in some cases, when the Kasai Procedure fails to be a success and liver problem worsens, liver transplantation is the next step within the first 1 to 2 years of life.
In recent years, the survival rate for liver transplantation has greatly improved. Thanks to the advancement of technology, patients who need the surgery need not rely on liver donors from small children. This was done in the past because it was important that the sizes match. Today, adult liver donors can be used and are called “reduced size” or “split liver” transplants.